scholarly journals Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Rheumatology ◽  
2019 ◽  
Vol 59 (7) ◽  
pp. 1684-1694 ◽  
Author(s):  
Veronika K Jaeger ◽  
Mohammed Tikly ◽  
Dong Xu ◽  
Elise Siegert ◽  
Eric Hachulla ◽  
...  

Abstract Objectives Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. Methods SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. Results The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. Conclusion Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality.

2013 ◽  
Vol 40 (6) ◽  
pp. 850-858 ◽  
Author(s):  
Xuli Jerry Zhang ◽  
Ashley Bonner ◽  
Marie Hudson ◽  
Murray Baron ◽  
Janet Pope ◽  
...  

Objective.Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc) and causes death. Once lung fibrosis occurs, disease course may become stable or decline. Little is known about risks for progression. We studied SSc–gastroesophageal (GE) involvement in relation to worsening forced vital capacity (FVC) on pulmonary function tests (PFT) to investigate whether it was related to progression. Our objective was to determine whether GE reflux and dysphagia are associated with progressive moderate/severe ILD as measured by PFT over 3 years.Methods.The Canadian Scleroderma Research Group is a multicenter SSc database that collects data annually. Using indicators of GE involvement and annual PFT, comparisons were made between no/mild ILD, stable moderate/severe ILD, and progressive moderate/severe ILD groups based on changes of FVC. Multivariate analyses determined associations between GE factors and ILD development and progression.Results.There were 1043 patients with SSc (mean age 55.7 yrs, mean disease duration 10.8 yrs); one-quarter had pulmonary fibrosis on chest radiograph that was related to FVC percentage predicted (Spearman’s rho −0.39; p < 0.01). Physician indicators such as esophageal dysmotility (p = 0.009) and postesophageal dilatation (p = 0.041), and patient indicators such as difficulty swallowing (p = 0.016) and waking up choking (p = 0.026) were associated with low FVC. In comparing progressive and stable moderate/severe FVC (< 70% predicted), early satiety (p = 0.018) and a combination term of postdilatation and choking (p = 0.042) increased risk of progression of ILD. Topoisomerase I was not associated with progression over followup.Conclusion.Symptoms of esophageal dysmotility were associated with worsening FVC in SSc, especially if both need for esophageal dilatation and choking were present.


2020 ◽  
Vol 63 (8) ◽  
pp. 2597-2608
Author(s):  
Emily N. Snell ◽  
Laura W. Plexico ◽  
Aurora J. Weaver ◽  
Mary J. Sandage

Purpose The purpose of this preliminary study was to identify a vocal task that could be used as a clinical indicator of the vocal aptitude or vocal fitness required for vocally demanding occupations in a manner similar to that of the anaerobic power tests commonly used in exercise science. Performance outcomes for vocal tasks that require rapid acceleration and high force production may be useful as an indirect indicator of muscle fiber complement and bioenergetic fitness of the larynx, an organ that is difficult to study directly. Method Sixteen women (age range: 19–24 years, M age = 22 years) were consented for participation and completed the following performance measures: forced vital capacity, three adapted vocal function tasks, and the horizontal sprint test. Results Using a within-participant correlational analyses, results indicated a positive relationship between the rate of the last second of a laryngeal diadochokinesis task that was produced at a high fundamental frequency/high sound level and anaerobic power. Forced vital capacity was not correlated with any of the vocal function tasks. Conclusions These preliminary results indicate that aspects of the laryngeal diadochokinesis task produced at a high fundamental frequency and high sound level may be useful as an ecologically valid measure of vocal power ability. Quantification of vocal power ability may be useful as a vocal fitness assessment or as an outcome measure for voice rehabilitation and habilitation for patients with vocally demanding jobs.


Pneumologie ◽  
2017 ◽  
Vol 71 (S 01) ◽  
pp. S1-S125
Author(s):  
U Costabel ◽  
C Albera ◽  
KU Kirchgaessler ◽  
F Gilberg ◽  
U Petzinger ◽  
...  

2021 ◽  
pp. 021849232110100
Author(s):  
Neetika Katiyar ◽  
Sandeep Negi ◽  
Sunder Lal Negi ◽  
Goverdhan Dutt Puri ◽  
Shyam Kumar Singh Thingnam

Background Pulmonary complications after cardiac surgery are very common and lead to an increased incidence of post-operative morbidity and mortality. Several factors, either modifiable or non-modifiable, may contribute to the associated unfavorable consequences related to pulmonary function. This study was aimed to investigate the degree of alteration and factors influencing pulmonary function (forced expiratory volume in one second (FEV1) and forced vital capacity), on third, fifth, and seventh post-operative days following cardiac surgery. Methods This study was executed in 71 patients who underwent on-pump cardiac surgery. Pulmonary function was assessed before surgery and on the third, fifth, and seventh post-operative days. Data including surgical details, information about risk factors, and assessment of pulmonary function were obtained. Results The FEV1 and forced vital capacity were significantly impaired on post-operative days 3, 5, and 7 compared to pre-operative values. The reduction in FEV1 was 41%, 29%, and 16% and in forced vital capacity was 42%, 29%, and 19% consecutively on post-operative days 3, 5, and 7. Multivariate analysis was done to detect the factors influencing post-operative FEV1 and forced vital capacity. Discussion This study observed a significant impairment in FEV1 and forced vital capacity, which did not completely recover by the seventh post-operative day. Different factors affecting post-operative FEV1 and forced vital capacity were pre-operative FEV1, age ≥60, less body surface area, lower pre-operative chest expansion at the axillary level, and having more duration of cardiopulmonary bypass during surgery. Presence of these factors enhances the chance of developing post-operative pulmonary complications.


2021 ◽  
pp. 239719832110340
Author(s):  
Yasser A Radwan ◽  
Reto D Kurmann ◽  
Avneek S Sandhu ◽  
Edward A El-Am ◽  
Cynthia S Crowson ◽  
...  

Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% ( p = 0.06), and any rhythm disorder was 18% versus 13% ( p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.


2021 ◽  
Vol 22 (sup1) ◽  
pp. 14-21
Author(s):  
Gabriela M. Stegmann ◽  
Shira Hahn ◽  
Cayla J. Duncan ◽  
Seward B. Rutkove ◽  
Julie Liss ◽  
...  

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